Polycystic Kidney Disease

What is polycystic kidney disease (PKD)?

Polycystic kidney disease (PKD) is a condition that causes cysts filled with fluid to grow on the kidneys. Over time, the cysts can damage the kidneys and lead to kidney failure. Polycystic kidney disease is the fourth leading cause of kidney failure in the United States.

There are three main types of PKD:

• Autosomal dominant PKD is the most common type of PKD. It is inherited from a parent who has PKD.
• Autosomal recessive PKD is a rare type of PKD. It is inherited from both parents, but the parents do not have symptoms. It is more commonly diagnosed in children.
• Acquired cystic kidney disease can develop in people who have long-term kidney problems or kidney failure and have been on dialysis for a long time. For this reason, it usually does not occur until later in life.

What are the signs and symptoms of polycystic kidney disease?

The symptoms of PKD can vary depending on the type your child has.

Many people with autosomal dominant PKD don't develop symptoms until they are adults, but symptoms can start in childhood. Symptoms may include:

• Stomach pain
• High blood pressure
• Easy bruising
• Urinary tract infections
• Blood in the urine
• Fluttering or pounding feeling in the chest

Children with autosomal recessive PKD may be diagnosed before birth. After birth, common symptoms include:

• High blood pressure
• Frequent urination
• Low blood cell counts
• Urinary tract infections

Usually, the earlier a child is diagnosed, the more severe their condition. Children born with autosomal recessive PKD may develop kidney failure within a few years after birth.

What causes polycystic kidney disease?

In most cases. autosomal dominant PKD and autosomal recessive PKD are both inherited from one or both parents. In very few cases, these types of PKD can occur with no family history. Acquired cystic kidney disease is caused by having long-term kidney problems or kidney failure.

How is polycystic kidney disease treated?

Treatment may depend on the specific type of PKD your child has and their individual symptoms. Your team at Arkansas Children's is experienced in the treatment of all types of PKD in children and will work with you to come up with the best plan for your child. Treatment options may include:

• Medicines to treat high blood pressure or urinary tract infections.
• Dietary changes may include increased water intake.
• Dialysis can help to clean the blood when the kidneys no longer work. There are two types of dialysis: hemodialysis, which is usually done in a dialysis center, and peritoneal dialysis, which is usually done at home. Your child's pediatric nephrologist will help you decide which type of dialysis is best for your child.
• A kidney transplant may be an option for children with kidney failure. The best match is from a parent or other relative. If no relative is a good match, your child may need to wait for a donor kidney. As the only certified program in the state approved to perform kidney transplants in children, your care team at Arkansas Children's can help your family through each step of the process.